1. Harika Yalamanchili PGY-3
PML
Harika Yalamanchili
PGY-3

2. Progressive multifocal leukoencephalopathy
Severe demyelinating disease of the CNS caused by reactivation of the JC polyomavirus
Asymptomatic primary infection occurs in childhood and antibodies can be found in 86% of adults
The virus remains latent in kidneys and lymphoid organs unless development of profound cellular immunosuppression where it reactivates and spreads to the brain
Induces a lytic infection of oligodendrocytes, CNS myelin- producing cells

3. Manifestations Symptoms vary and depend on location of lesions
Typically spares the optic nerves and the spinal cord
Subacute neurologic deficits
AMS
Motor deficits (hemiparesis or monoparesis)
Limb ataxia
Gait ataxia
Visual symptoms (hemianopia or diplopia)
Aphasia
Seizures

4. Imaging
Symmetric or asymmetric multifocal areas of white matter demyelination that do not conform to cerebrovascular territories and exhibit neither mass effect nor contrast enhancement
CT: hypodense patchy or confluent white matter regions
MRI: lesions with decreased signal intensity on T1, T2, and FLAIR with hyperintensity on diffusion-weighted sequences
Bilateral and localized preferentially to the periventricular areas and the subcortical white matter

5. Diagnosis
Diagnosis
Gold standard is brain biopsy
Histopathologic triad of demyelination, bizarre astrocytes, and enlarged oligodendroglial nuclei
Compatible clinical and neuroimaging features + JC virus DNA in the CSF
Differential includes HIV encephalopathy, primary CNS lymphoma, CNS vasculitis, reversible posterior leukoencephalopathy, and VZV encephalopathy

6. Treatment No specific treatment Initiate or optimize ART
High risk for IRIS
Withdraw immunosuppressive agents if possible
Progressive and fatal
Non-HIV: 3 months
HIV without ART: 4-5 months
HIV with ART: 1.8 years